Mohtashami Maryam, Razavi Azadehsadat, Abolhassani Hassan, Aghamohammadi Asghar, Yazdani Reza
Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran.
Research Center for Immunodeficiencies, Tehran University of Medical Sciences, Tehran, Iran.
Int Rev Immunol. 2022;41(2):135-159. doi: 10.1080/08830185.2020.1868454. Epub 2021 Jan 19.
Primary immunodeficiency (PID) or Inborn errors of immunity (IEI) refers to a heterogeneous group of disorders characterized by immune system impairment. Although patients with IEI manifest highly variable symptoms, the most common clinical manifestations are recurrent infections, autoimmunity and malignancies. Some patients present hematological abnormality including thrombocytopenia due to different pathogenic mechanisms. This review focuses on primary and secondary thrombocytopenia as a complication, which can occur in IEI. Based on the International Union of Immunological Societies phenotypic classification for IEI, the several innate and adaptive immunodeficiency disorders can lead to thrombocytopenia. This review, for the first time, describes manifestation, mechanism and therapeutic modalities for thrombocytopenia in different classes of IEI.
原发性免疫缺陷（PID）或先天性免疫缺陷（IEI）是指一组以免疫系统受损为特征的异质性疾病。尽管IEI患者表现出高度可变的症状，但最常见的临床表现是反复感染、自身免疫和恶性肿瘤。一些患者由于不同的致病机制出现血液学异常，包括血小板减少。本综述重点关注作为并发症可发生在IEI中的原发性和继发性血小板减少。基于国际免疫学会联盟对IEI的表型分类，几种先天性和适应性免疫缺陷疾病可导致血小板减少。本综述首次描述了不同类型IEI中血小板减少的表现、机制和治疗方式。
